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Haematology (E) > Lymphoma 1 > Flashcards

Lymphoma 1 Flashcards

1
Q

What is lymphoma?

A

Neoplastic (malignant) tumour of lymphoid cells

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2
Q

Where are lymphomas usually found?

A

Lymph nodes, bone marrow and/or blood (lymphatic system)
Lymphoid organs; spleen or the gut-associated lymphoid tissue
Skin (often T cell disease)

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3
Q

What is the proportion of Hodgkin vs Non-Hodgkin lymphoma?

A

Non-Hodgkin Lymphoma - 80%

Hodgkin Lymphoma - 20%

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4
Q

What is the relationship between lymphomas and risk factors?

A

Most lymphoma subtypes/cases are sporadic with no known risk factors
Some lymphoma subtypes have specific risk factors - immune diseases acquired or iatrogenic, associated specific infections or inflammation

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5
Q

What is the reason for existing ‘limited’ DNA instability of lymphocytes?

A 
For an adaptive immune response
Generates immunoglobulin and T cell receptor diversity and Ig class switching
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6
Q

What happens to lymphocytes to generate antibody diversity?

A

Cut and recombined

Subjected to deliberate DNA mutagenesis (somatic hypermutation)

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7
Q

What is the benefit of rapid cell proliferation in the germinal centre?

A

Allows rapid response to infection

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8
Q

What is the downside to rapid cell proliferation in the germinal centre?

A

Rapid multiple cell divisions = increased chance of DNA replication errors

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9
Q

What is the benefit for lymphocytes being dependent on apoptosis?

A

Exquisite antibody specificity & eliminates self reactive clones

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10
Q

What is the downside to lymphocytes dependent on apoptosis?

A

Apoptosis is ‘switched off’ in germinal centre

Consequences of mutations in apoptosis regulating genes

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11
Q

What happens in the t(8;14) gene recombination error?

A

C-MYC oncogenes are located downstream of the IgH promoter (instead of the Ig Heavy chain gene) and are over expressed

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12
Q

What are the main groups/mechanisms of the risk factors for certain NHL subtypes?

A 
Constant antigenic stimulation
Viral infection (direct viral integration of lymphocytes)
Loss of T cell function and EBV infection plus EBV infections of B cells
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13
Q

What can cause constant antigenic stimulation?

A 
Bacterial infection (chronic)
Auto immune disorders
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14
Q

What can cause loss of T cell function and EBV infection plus EBV infections of B cells?

A

Loss of T cells (untreated HIV infection)

Iatrogenic immunosuppression

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15
Q

What lymphoma can arise as a result of bacterial or auto immune antigenic drive?

A

B cell Non Hodgkin Lymphoma Marginal zone subtype (MZL)

Enteropathy associated T-Cell Non Hodgkin lymphoma (EATL)

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16
Q

What bacterial infections can cause MZL?

A

H. pylori - Gastric MALT (mucosa associated lymphoid tissue, MZL of stomach)
Sjogren syndrome (MZL of salivary glands
Hashimoto’s (MZL of thyroid)

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17
Q

What can cause EATL?

A

Coeliac disease/Gluten: small intestine EATL

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18
Q

What is an example of direct viral integration and lymphomagenesis?

A

HTLV1 retrovirus infects T cells by vertical transmission

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19
Q

What infection is associated with loss of T cell function lymphoma?

A

EBV infection

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20
Q

What are the elements of the lymphoreticular system?

A

Generative LR tissue
Reactive LR tissue
Acquired LR tissue

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21
Q

Give examples of generative lymphoreticular tissue and their function

A

Bone marrow and thymus

Generation/maturation of lymphoid cells

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22
Q

Give examples and function of reactive lymphoreticular tissue

A

Lymph nodes and spleen

Development of immune reaction

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23
Q

Give examples and function of acquired lymphoreticular tissue

A

Extranodal lymphoid tissue e.g. skin, stomach, lungs

Development of local immune reaction

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24
Q

Summarise the role of B cells

A

Express surface immunoglobulin

Antibody production

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25
Q

Summarise the role of T lymphocytes

A

Express surface T cell receptor
Regulation of B cells and macrophage function
Cytotoxic function

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26
Q

What can be found in the mantle zone of the lymphoid follicle?

A

Naïve unstimulated B cells

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27
Q

What can be found in the germinal centre of the lymphoid follicle?

A

B cells

Antigen presenting cells

28
Q

What comprises the T cell area?

A

T cells
Antigen presenting cells
High endothelial vessels

29
Q

How are types of lymphocytes detected?

A

Detected in tissue samples using immunohistochemistry
Identify subtypes and different stages of development by the different types of cell surface receptors expressed by the cells

30
Q

Define lymphoma

A

Neoplastic proliferation of lymphoid cells forming discrete tissue masses

31
Q

What is the site of lymphoma?

A

Arise in and involve lymphoid tissues (including acquired lymphoid tissue - extranodal lymphomas)

32
Q

What are the various pathogenic causes of lymphoma?

A

Inherent instability of lymphoid cells
Inherited disorders increasing instability
Viral agents (EBV, HTLV-1)
Environmental agents (mutagens, chronic immune stimulation)
Iatrogenic causes (chemotherapy, radiotherapy)

33
Q

What are the types of Hodgkin lymphoma?

A

Classical

Lymphocyte predominant

34
Q

Which cell type stains CD20?

A

B cell

35
Q

Give examples of low grade B cell Non-Hodgkin lymphomas

A

Follicular lymphoma
Small lymphocytic lymphoma / chronic lymphocytic leukaemia
Marginal zone lymphoma

36
Q

Give examples of high grade B cell non-Hodgkin lymphoma

A

Diffuse large B cell lymphoma

Burkitt’s lymphoma

37
Q

Give an example of an aggressive B cell Non-Hodgkin lymphoma

A

Mantle cell lymphoma

38
Q

Which cell type stains CD3 and CD5?

A

T cell

39
Q

How does follicular lymphoma present?

A

Disease of middle age/elderly

Lymphadenopathy

40
Q

What is the histopathology of follicular lymphoma?

A

Follicular pattern

Germinal centre cell origin CD10, bcl-6+

41
Q

What are the molecular features of follicular lymphoma?

A

14;18 translocation involving bcl-2 gene

42
Q

How do you make an immunohistochemical diagnosis of follicular lymphoma?

A

Detection of bcl-2

43
Q

What are the clinical features of small lymphocytic lymphoma / CLL?

A

Middle age / elderly

Nodes - lymphadenopathy, or blood - high white count

44
Q

What is the histopathology of small lymphocytic lymphoma / CLL?

A

Small lymphocytes, naïve or post-germinal centre memory B cell
CD5, CD23+

45
Q

What do you call the transformation of small lymphocytic lymphoma / CLL to high grade lymphoma?

A

Richter transformation

46
Q

Where and why does marginal zone lymphoma arise?

A

Extranodal sites (gut, lung spleen) in response to chronic antigen stimulation

47
Q

What are the clinical features of mantle cell lymphoma?

A

Middle age, male predominance
Lymph nodes, GI tract
Disseminated disease at presentation

48
Q

What is the histopathology of mantle cell lymphoma?

A

Located in mantle zone
Pre-germinal centre cell
Aberrant CD5, cyclin D1 expression

49
Q

What are the molecular features of mantle cell lymphoma?

A

11;14 translocation

Cyclin D1 over expression

50
Q

What are the clinical features of Burkitt’s lymphoma?

A

Jaw or abdominal mass children/young adults
Endemic, sporadic, immunodeficiency
EBV associated

51
Q

What is the histopathology of Burkitt’s lymphoma?

A

Germinal cell centre origin

Starry sky appearance

52
Q

What are the molecular features of Burkitt’s lymphoma?

A

C-myc translocation (8:14, 2:8, 8;22)

53
Q

What are the clinical features of diffuse large B cell lymphoma?

A

Middle age / elderly

Lymphadenopathy

54
Q

What is the histopathology of diffuse large B cell lymphoma?

A

Germinal centre or post-germinal centre B cell
Sheets of large lymphoid cells
Germinal centre phenotype = good prognosis
P53 positive, high proliferation fraction = poor prognosis

55
Q

What are the features of peripheral T cell lymphoma?

A 
MA / elderly
Lymphadenopathy and extranodal sites
Large T lymphocytes
Often with associated reactive cell population, esp eosinophils
Aggressive
56
Q

What are the clinical features of anaplastic large cell lymphoma?

A

Children / young adults

Lymphadenopathy

57
Q

What are the histopathology features of anaplastic large cell lymphoma?

A

Large epithelioid lymphocytes

T cell or null phenotype

58
Q

What are the molecular features of anaplastic large cell lymphoma?

A

2;5 translocation

Alk-1 protein expression

59
Q

What are the subtypes of classical Hodgkin’s lymphoma?

A

Nodular sclerosing
Mixed cellularity
Lymphocyte rich and lymphocyte depleted

60
Q

What are the clinical features of classical HL?

A

Young and MA

Often involves just single lymph node group

61
Q

What are some key features of classical HL?

A

Thought to be germinal centre/post-germinal centre B cell origin
EBV associated
CD30+, CD15+, CD20-

62
Q

What is the histopathology of classical HL?

A

Sclerosis, mixed cell population in which Reed-Sternberg and Hodgkin cells with eosinophils

63
Q

What is the key clinical feature of nodular LP Hodgkins Lymphoma?

A

Isolated lymphadenopathy

64
Q

What are some other features of nodular LP Hodgkins Lymphoma?

A

Germinal centre B cell (positive for some germinal centre B cell markers)
No association with EBV
CD20+, CD30-, CD15-
Indolent, can transform to high grade B cell lymphoma

65
Q

What is the histopathology of nodular LP Hodgkins lymphoma?

A

B cell rich nodules with scattered L&H cells

Haematology (E) (14 decks)

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